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Neo boards > ID and Immunology > Flashcards

ID and Immunology Flashcards

1
Q

Disorder of neutrophil function

  • recurrent infection esp of SKIN, coarse facial features, broad nasal bridge, ECZEMA
A

Hyperimmunoglobulin E (abnormal neutrophil chemotaxis - STAT3 mutation)

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2
Q

Recurrent bacterial infections
Poor wound healing
No pus formation
Omphalitis
Delayed separation of umb cord (> 21 days)

A

Leukocyte adhesion deficiency

  • increased nuetrophils but defective adhesion and migration
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3
Q

Increased risk of fungal infection esp if immune system already compromised

A

Myeloperoxidase deficiency

  • most common immunodeficiency
  • can be silent
  • recurrent candidal infections
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4
Q

Recurrent infections
Peripheral neuropathy
Oculocutaneous albinism
Nystagmus

finding: wbc inclusions

A

Chediak Higashi

  • abnormal neutrophil degranulation

wbc inclusions -giant intracellular granules

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5
Q

Increased risk of abscesses
Poor wound healing
Granuloma formation
Recurrent pseudomonas, listeria, staph, E.coli infections

note: X linked mostly

A

Chronic granulomatous disease

  • dysfunctional NADPH oxidase and abnormal phagocytic ability
  • catalase + organisms (cats Need PLACESS to Belch their Hairballs)
  • normal B and T cell function

Dx - NBT test (+ = do NOT see the resp burst response)

Nocardia, pseudomonas, listeria, aspergillus, candida, E. coli, staphylococcus, serratia, B. cepacia and H. pylori.

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6
Q

Bone marrow dysfunction
Dysmorphic features
Steatorrhea with FTT (malabsorption)
Skeletal defects

  • increased risk of myelodysplastic syndromes
A

Shwachman-Diamond Syndrome

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7
Q

No B cells
Profoundly low immunoglobins, and no IgM
Low total protein
Recurrent sinopulmonary infections

LN tissue -> no stain for B cells

A

X linked agammaglobulinemia

Rx - antibody replacement, IVIG q3-4wks, IgG > 500 goal

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8
Q
  • Recurrent peri-rectal abscess and oral ulcers
  • Episodes of infection with PCP,salmonella, crypto
  • Low/no IgG, IgA, IgE
  • normal or high IgM
A

HyperIgM

XL recessive

note: reported in association with congenital rubella

Rx - antibody replacement, IVIG q3-4wks, IgG > 500 goal

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9
Q

Non-primary immunodeficiencies (Describe)

  • G6PD
  • Galactosemia
  • Glycogen storage disorder 1B
  • Trisomy 21
  • Turners syndrome
  • TORCH
A

Decreased NADPH activity - decreased oxygen dependent killing

Inhibitory effects of galactose on PMNs

Neutropenia

Decreased chemotactic activity
Low IgG, IgM, IgA

Low IgG, IgM

Impair antibody production and function

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10
Q

No thymus on XR
Viral infections, fungal infections
Mildly low ALC
Cardiac
Hypocalcemia (hypoPTH)
Cleft palate

A

22q11.2 deletion, DiGeorge

CATCH 22

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11
Q

Eczema
Thrombocytopenia
Immunodeficiency

Low plts
Decreased IgM
Increased IgA, IgE
Normal IgG
Decreased lymphocytes

A

Wiskott Aldrich syndrome

X linked inheritance

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12
Q

Perinatal HIV

clinical presentation

immune cell counts

A

Most common global form of combined immune deficiency in neonates

*Clinical presentation ranges from asymptomatic to mucocutaneous candidiasis, splenomegaly, lymphadenopathy, lymphopenia, thrombocytopenia

*Elevated IgA and IgElevels *Decreased CD4 cells, normal number CD8

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13
Q

SCID

A

*Deficiency of both antibody and cell-mediated immunity (low/normal # B cells, decreased T cells/NK cells)

*Diarrhea, pneumonia, otitis, sepsis, cutaneous infections, eosinophilia

*CMV, PCP, Gram negative sepsis, mucocutaneous candidiasis

*GVHD–following blood transfusion–20-30% risk of maternal cell-mediated GVHD (placental transfer of maternal T cells)

10 different mutations
Can be XL inheritance

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14
Q

How to diagnose SCID.

Management - early is key.

A

Dx - newborn screen, low TREC (T cell excision circle - means low T cells)

*Bone marrow transplant *Appropriate enzyme replacement therapy
* Irradiated blood products only
* IVIg if combined defect
*PCP prophylaxis
*No live vaccines

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15
Q

Severe watery diarrhea, FTT, dermatis, type 1 DM

Eosinophilia, high IgE levels

A

Immunodysreguation, Polyendocrinopathy,
Enteropathy

Presents in neonatal period

Mutation in forkheadbox protein 3 located on the X chromosome

Impaired TREGsuppressor function Decreased IL-2 and IFN-γproduction

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16
Q

Match organism with type of precaution for infection control

A
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17
Q

Review and provide thoughts on management for different scenarios (infant at risk congenital syphilis)

A
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18
Q

Transmission pattern - CMV, rubella, toxo,

A
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19
Q

When is baby at greatest risk of varicella infection from mother

A

2 days before and 5 days after maternal disease -> at HIGH risk so infant should get varicella immunoglobulins (passive immunity?) within 10 days. Precautions for 21 - 28days (? confirm).

IF congenital varicella - likely occurred much earlier in utero - so no precaution (as rash would be cicatricial - not active transmission)

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20
Q

Mother with active varicella infection (seronegative mother) - how to treat mother

A
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21
Q

Recommendations for zidovudine post-natally – for < 35 weeks and for > 35 weeks

A
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22
Q

Recommendations for nevirapine post-natally

A
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23
Q

Review - decreased function of neutrophils among premature infants

A
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24
Q

Neutrophils

  • NB vs adults
  • Differences in preterm
A
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25
Q

Complement review

A
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26
Q

Review immunoglobulins - fetal, newborn

A
27
Q

B cells - newborns vs adults
- diff in preterm?

A
28
Q

Immunodeficiency in DiGeorge

A
29
Q

CMV vs congenital rubella

A
30
Q

The rash of congenital syphilis

A
31
Q

Complement system

A

decreased classical pathway in NB (increased risk pseudomonas); adult 6 - 18 mo

32
Q

Neonatal neutrophils

A

Decreased function - less able to get out of bloodstream(poor diapadesis), poor phagocyte killing (more susc GN), decreased chemotaxis

33
Q

Monocytes and macs

A

Poor response to interferon gamma
Make less cytokines until 12 mo
low mono for 1st 5 days
poor antigen presentation until 12 mo

34
Q

ADAPTIVE IMMUNE SYSTEM

4 to 7 days to develop

A

NB Bcells - mostly differentiate to IgM

35
Q

Germinal matrix center

A

absent infetus - not until antigenic stimulation

36
Q

B cells

A

Decreased IgG in preterm andFGR
High at birth IgG from mother, nadir 2 - 4 months, until 9 mo but may not completely clear until 18 months

IgA - from colostrom

IgM - what a newborn is mostly making

37
Q

T cells

A

CD4
CD8 - less cytokine production inNB, limited cytotoxic activity

38
Q

Immunoglobulin by age

A

Adult levels of IgM by 1 yo - 75%

20% of adult levels by 1 yo - IgA

39
Q
A

C botulinum - gram positive rod

40
Q

CMV

A

CMV E

41
Q

Infections stillbirths

A
42
Q

cong syphilis

A
43
Q

HIV and breastfeeding

A
44
Q

CONS

A

C - surveillance not help because ubiquotous skin colonizer

45
Q

Early onset listeria

A
46
Q

Measures that have reduced HIV MTCT

A
47
Q

Presumptive HIV neg in infant

A
48
Q

ANC and I:T how to use them

A
49
Q

CONS prevention, RFs

A
50
Q

CONS prevention

A
51
Q

Hep B serologies

A
52
Q

Osteo - tricky facts

A
53
Q

Osteo tricky facts

A
54
Q

DiGeorge immunodef

A
55
Q
A
56
Q
A
57
Q
A
58
Q

CLABSI greatest RFs

A
59
Q
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60
Q
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61
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62
Q
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63
Q
A

Neo boards (14 decks)

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